Persistent fetal vasculature: ocular features, management of cataract and outcomes

Purposes: To describe ocular features, management of cataract and functional outcomes in patients with persistent fetal vasculature.

Methods: Retrospective, descriptive case series of patients with persistent fetal vasculature. Data were recorded from the Congenital Cataract Section of Federal University of São Paulo, Brazil from 2001 to 2012. All patients were evaluated for sex, age at diagnosis, systemic findings, laterality, age at surgery, and initial and final follow-up visual acuities. Follow-up and complications after cataract surgery were recorded. Ultrasound was performed in all cases and ocular eco-Doppler was performed in most.

Results: The study comprised 53 eyes from 46 patients. Age at diagnosis ranged from 5 days of life to 10 years-old (mean 22.7 months). Twenty-seven patients were male (58.7%). Persistent fetal vasculature was bilateral in 7 patients (15.2%). Forty-two eyes (79.2%) had combined (anterior and posterior forms) PFV presentation, 5 eyes (9.4%) had only anterior persistent fetal vasculature presentation and 6 eyes (11.3%) had posterior persistent fetal vasculature presentation. Thirty-eight eyes (71.7%) were submitted to cataract surgery. Lensectomy combined with anterior vitrectomy was performed in 18 eyes (47.4%). Phacoaspiration with intraocular lens implantation was performed in 15 eyes (39.5%), and without lens implantation in 5 eyes (13.2%). Mean follow-up after surgery was 44 months. Postoperative complications were posterior synechiae (3 cases), retinal detachment (2 cases), phthisis (3 cases), posterior capsular opacification (8 cases), inflammatory pupillary membrane (5 cases), glaucoma (4 cases), intraocular lens implantation displacement (1 case) and vitreous hemorrhage (2 cases). Complications were identified in 19 (50%) of the 38 operated eyes. Visual acuity improved after cataract surgery in 83% of the eyes.

Conclusions: Patients with persistent fetal vasculature have variable clinical presentation. There is an association of persistent fetal vasculature with congenital cataract. Severe complications are related to cataract surgery in patients with persistent fetal vasculature, but 83% of the operated eyes improved visual acuity.